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Blood Transfus. 2010 July; 8(3): 211.
PMCID: PMC2906196

Bone marrow necrosis in sickle cell anaemia

Dear Editor,

The paucity of descriptions of bone marrow necrosis in patients with sickle cell anaemia is not a reflection of the low rate of this condition, but rather the low frequency of examination of bone marrow in painful areas during crises.

A 24-year old Greek female with sickle cell anaemia presented with an acute painful crisis. On physical examination she was ill-looking, febrile, jaundiced and had a palpable liver. Laboratory data showed leucocytosis (white blood cell count: 30 x 109/L) with a predominance of polymorphonuclear leuocyctes and left shift, mild thrombocytosis (platelet count: 523 x 109/L) and anaemia, with a decrease in haemoglobin concentration from 8.5 to 7 g/dL in the 24 hours after admission. Sickled erythrocytes, target cells and a leucoerythroblastic picture were evident on inspection of the blood film. The woman’s serum bilirubin concentration on admission was 3.54 mg/dL (normal range: 0.5–1.2 mg/dL) and progressively declined thereafter; the lactate dehydrogenase concentration was 900 U/L (normal range: 200–460 U/L). Alkaline phosphatase levels were above normal (600 U/L; normal range: 64–260 U/L) and peaked (1208 U/L) on the third day of hospitalisation. Parvovirus B19 infection was excluded by means of serology and quantitattive polymerase chain reactioin (Roche LightCycler parvovirus B19 quantification kit). Clinical and laboratory data suggested bone marrow necrosis. Technetium-99m sulphur colloid bone marrow imaging showed absence of functioning bone marrow in both iliac regions (Figure 1A). A bone marrow biopsy from the iliac crest demonstrated extensive destruction of haematopoietic tissue, including the stroma, with preservation of the bone, all compatible with bone marrow necrosis (Figure 1B).

Figure 1
A. Technetium-99m sulphur colloid scintigraphy of the bone marrow. Absence of tracer uptake in both iliac regions

Bone marrow necrosis may be seen as a complication in patients with leukaemia, malignancy and sickle cell disease. In this last condition, a veno-occlusive crisis, leucoerythroblastosis and increased lactate dehydrogenase are typically present. The diagnosis does, however, require a bone marrow biopsy1. Technetium-99m sulphur colloid scintigraphy can locate the area of necrosis, as decreased tracer uptake is seen on the bone marrow scan 2.

References

1. Ataga KI, Orringer EP. Bone marrow necrosis in sickle cell disease: a description of three cases and a review of the literature. Am J Med Sci. 2000;320:342–72. [PubMed]
2. Kim SK, Miller JH. Natural history and distribution of bone and bone marrow infarction in sickle hemoglobinopathies. J Nucl Med. 2002;43:896–900. [PubMed]

Articles from Blood Transfusion are provided here courtesy of SIMTI Servizi